We report the first comparison of parent- versus child-completed CHQ findings for children who have undergone the Fontan procedure. Compared with their children’s perceptions, parents reported significantly lower scores (worse functioning) for their children in the domains of physical functioning, impact on school or activities from emotional and behavioral problems, impact on school or activities from physical health issues, general behavior, mental health, self-esteem, and general health perceptions. In explorations of factors that might explain differences in child/parent pairs for the domains of physical functioning and impact of physical limitations, in general the associations were weak. For the physical limitations domain, lower parent- versus child-reported scores were associated with anatomic features such as pulmonary artery anomalies at the time of the Fontan operation and fenestration at the time of the Fontan operation. Lower parent- versus child-reported scores also were associated with the presence of multiple noncardiac medical conditions in the children.
Among patients with CHDs, those with a single ventricle who have undergone the Fontan procedure are among those most likely to have multiple morbidities. These morbidities include myocardial dysfunction, diminished exercise capacity, arrhythmias, protein-losing enteropathy, thrombotic complications, somatic growth retardation, and adverse neurodevelopmental outcomes.2,4,14
Certain noncardiac morbidities, such as asthma and nonasthma respiratory problems, allergies, orthopedic problems, sleep and speech problems, anxiety, developmental delays, and learning and behavior problems, have been identified as having an impact on functional health status.3,15
As this population reaches adolescence and adulthood, it becomes increasingly important to understand the impact of these comorbidities on functional outcomes and quality of life, to design useful interventions.
The terminology used to describe functional health status and health-related quality of life has been inconsistent in the CHD literature. Gill and Feinstein16
defined quality of life as follows: “Rather than being a description of patient’s health status, quality of life is a reflection of the way that patients perceive and react to their health status and to other, nonmedical aspects of their lives.” In a review of 70 articles published in the medical literature, using the 10 criteria developed by Gill and Feinstein,16
Moons et al17
found that “quality of life assessments in patients with CHDs showed major conceptual and methodological drawbacks.” They also found that the term “quality of life” often was used inappropriately. This observation was supported by several articles in which the authors drew conclusions about quality of life although it was not specifically measured. 17
The CHQ measures the magnitude of perceived overall functioning as affected by health-related issues, which we have equated with the term health status.3
Functional health status is subjective and should be assessed as perceived by patients themselves whenever possible.18
Few studies have examined differences between parent and child reports of functional health status.8,19–27
Similar to our findings, other studies reported a tendency for the parents of children with serious or chronic illnesses to report poorer health status for their children than the children reported for themselves. Britto et al8
suggested that differences may reflect a generally more optimistic future outlook by adolescents than by their parents or perhaps less-than-full disclosure of prognoses to the adolescents. Havermans et al24
evaluated 36 patients by using a validated cystic fibrosis questionnaire. Their findings were similar to those of Britto et al,8
in that the children reported their quality of life to be better than their parents reported, especially with regard to body image, emotional functioning, and treatment burden. The authors concluded that children may “adapt better to their situation and rate the impact of the illness to be less.”24
Klassen et al25
evaluated the agreement between parent and children reports of quality of life for children with attention-deficit/hyperactivity disorder, by using the CHQ. They also demonstrated a more-optimistic outlook for the adolescents, compared with their parents.
Theunissen et al26
studied 1105 Dutch children by using the Netherlands Organization for Applied Scientific Research Academic Medical Center (TNO-AZL) Children’s Quality of Life questionnaire to evaluate the agreement between child and parent reports of children’s health-related quality of life. In contrast to our study, the children reported significantly lower health-related quality of life than did their parents. In a large study by Waters et al,27
2096 parent/adolescent dyads with or without illness were evaluated by using the CHQ, with the aim of examining the extent to which parents and their adolescent children agreed or disagreed regarding physical, emotional, mental, and social domains of health and well-being. That study also showed a less-optimistic evaluation by the adolescents, compared with their parents, for both normal adolescents and children diagnosed as having generic chronic illnesses.27
The authors found that the most disagreement was observed for family activities, body pain, general health, and mental health, with significantly lower health experienced by those with illnesses. In both of those studies,26,27
the authors concluded that large differences between child and parent reports could exist and “relying on parent report will only partly inform the clinician of the severity of health experienced.”27
In CHDs, overprotective parental behavior is not necessarily related to the severity of the lesion.22,28
Cheuk et al29
assessed parents’ knowledge about their child’s heart disease, treatment, and prevention of complications for relatively simple defects. That study suggested that parents have important knowledge gaps. Parent/child differences in their assessments of health status also may be attributable to the adolescents’ state of cognitive development, because they view themselves as relatively invulnerable.8
Given that these children have been living with CHDs their entire lives, they may have a different definition of “healthy” than their parents. However, parents have additional stressors related to feelings of distress and hopelessness and to financial concerns,22,30
which can adversely affect their perceived quality of life and perceptions about their child. Consideration must be given to a family’s ability to return to “normalcy” after cardiac surgery. Families that do not develop appropriate coping mechanisms may underestimate the child’s health after the Fontan procedure; the reverse may be true for families that are more resilient.
Certain limitations pertain to this study. We relied on the use of published normative data rather than simultaneously studying a cohort of normal control subjects. We had only cross-sectional data and could not explore temporal trends in health perceptions, such as whether children’s ratings become more like those of their parents as the children become adults. A significant limitation of this study is the use of a generic functional health status instrument, rather than one with a disease-specific module. Generic measurements have been useful in comparisons of patient groups with each other and with normal subjects. 19,23–27,30
However, Britto et al8
pointed out in their study “significant concerns about the comparability of the adolescent and parent forms of the CHQ,” which may contribute to differences in reports between parents and adolescents. These potential measurement issues related to the CHQ must be viewed as a limitation. Finally, we evaluated only children 10 to 18 years of age, because the CHQ is not applicable to children <10 years of age. Similarly, available normative data for the parent-completed CHQ span a patient age range larger than that for our study population, which might influence generalizability. Therefore, our results may not be generalizable to children <10 years of age.